Adult-onset Still's disease
Encyclopedia
Adult-onset Still's disease is a rare form of inflammatory arthritis
that was characterized by EG Bywaters in 1971. The underlying cause is unknown. It usually presents with high spiking fevers, joint and muscle pains, a salmon colored rash and other symptoms of systemic inflammation.
physician
Sir George Frederic Still
(1861–1941).
, elevated serum ferritin, a 'salmon-pink' rash, pyrexia and lymphadenopathy
. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. Patients experiencing a flare-up from Adult-onset Still's disease usually report extreme fatigue, swelling of the lymph glands, and less commonly fluid accumulation in the lungs and heart.
. At least seven sets of diagnostic criteria have been devised, however the Yamaguchi criteria have the highest sensitivity. Diagnosis requires at least five features, with at least two of these being major diagnostic criteria.
are used to treat severe symptoms of Still's. Other medications include hydroxychloroquine
, penicillamine
, azathioprine
, methotrexate
, etanercept
, anakinra
, cyclophosphamide
, adalimumab
, rituximab
, and infliximab
.
Adult-onset Still's disease is chronic; and reports of remission is either slim or temporary at best.
The condition "juvenile-onset Still disease" is now usually grouped under juvenile rheumatoid arthritis. However, there is some evidence that the two conditions are closely related.
Arthritis
Arthritis is a form of joint disorder that involves inflammation of one or more joints....
that was characterized by EG Bywaters in 1971. The underlying cause is unknown. It usually presents with high spiking fevers, joint and muscle pains, a salmon colored rash and other symptoms of systemic inflammation.
History
Still's disease is named after EnglishUnited Kingdom
The United Kingdom of Great Britain and Northern IrelandIn the United Kingdom and Dependencies, other languages have been officially recognised as legitimate autochthonous languages under the European Charter for Regional or Minority Languages...
physician
Physician
A physician is a health care provider who practices the profession of medicine, which is concerned with promoting, maintaining or restoring human health through the study, diagnosis, and treatment of disease, injury and other physical and mental impairments...
Sir George Frederic Still
Sir George Frederic Still
Sir George Frederic Still, KCVO was an English paediatrician and author of numerous medical textbooks and articles who first described a form of juvenile idiopathic arthritis which bears his name, as well as being the first to describe ADHD...
(1861–1941).
Epidemiology
Adult-onset Still's Disease is rare and has been described all over the world. Prevalence is estimated at 1.5 cases per 100,000-1,000,000 population. There is a bimodal age distribution with one peak incidence between ages 15-25 and a second peak between ages of 36-46 years.Signs and symptoms
The disease typically affects 16–35 year olds and presents with arthralgiaArthralgia
Arthralgia literally means joint pain; it is a symptom of injury, infection, illnesses or an allergic reaction to medication....
, elevated serum ferritin, a 'salmon-pink' rash, pyrexia and lymphadenopathy
Lymphadenopathy
Lymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. Patients experiencing a flare-up from Adult-onset Still's disease usually report extreme fatigue, swelling of the lymph glands, and less commonly fluid accumulation in the lungs and heart.
Diagnosis
The diagnosis is clinical, not based upon serologySerology
Serology is the scientific study of blood serum and other bodily fluids. In practice, the term usually refers to the diagnostic identification of antibodies in the serum...
. At least seven sets of diagnostic criteria have been devised, however the Yamaguchi criteria have the highest sensitivity. Diagnosis requires at least five features, with at least two of these being major diagnostic criteria.
| Major criteria | Minor criteria |
|---|---|
| Fever of at least 39C for at least one week | Sore throat |
| Arthralgias or arthritis for at least two weeks | Lymphadenopathy |
| Nonpruritic salmon colored rash (usually over trunk or extremities while febrile) | Hepatomegaly or splenomegaly |
| Leukocytosis ( 10,000/microL or greater), with granulocyte predominance | Abnormal liver function tests |
| Negative tests for antinuclear antibody and rheumatoid factor |
Treatment
Treatment for adult-onset Still's disease is with anti-inflammatory drugs. Steroids such as prednisonePrednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
are used to treat severe symptoms of Still's. Other medications include hydroxychloroquine
Hydroxychloroquine
Hydroxychloroquine is an antimalarial drug, sold under the trade names Plaquenil,Axemal, Dolquine, and Quensyl, also used to reduce inflammation in the treatment of rheumatoid arthritis and lupus...
, penicillamine
Penicillamine
Penicillamine is a pharmaceutical of the chelator class. It is sold under the trade names of Cuprimine and Depen. The pharmaceutical form is D-penicillamine, as L-penicillamine is toxic...
, azathioprine
Azathioprine
Azathioprine is a purine analogue immunosuppressive drug. It is used to prevent organ rejection following organ transplantation and to treat a vast array of autoimmune diseases, including rheumatoid arthritis, pemphigus, inflammatory bowel disease , multiple sclerosis, autoimmune hepatitis, atopic...
, methotrexate
Methotrexate
Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
, etanercept
Etanercept
Etanercept is a drug that treats autoimmune diseases by interfering with the tumor necrosis factor by acting as a TNF inhibitor. Pfizer describes in a SEC filing that the drug is used to treat rheumatoid, juvenile rheumatoid and psoriatic arthritis, plaque psoriasis and ankylosing spondylitis...
, anakinra
Anakinra
Anakinra is a drug used to treat rheumatoid arthritis.-Mechanism:Anakinra is an interleukin-1 receptor antagonist...
, cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
, adalimumab
Adalimumab
Adalimumab is the third TNF inhibitor, after infliximab and etanercept, to be approved in the United States. Like infliximab and etanercept, adalimumab binds to TNFα, preventing it from activating TNF receptors; adalimumab was constructed from a fully human monoclonal antibody, while infliximab...
, rituximab
Rituximab
Rituximab, sold under the trade names Rituxan and MabThera, is a chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of B cells...
, and infliximab
Infliximab
Infliximab is a monoclonal antibody against tumour necrosis factor alpha . It is used to treat autoimmune diseases. Remicade is marketed by Janssen Biotech, Inc...
.
Adult-onset Still's disease is chronic; and reports of remission is either slim or temporary at best.
The condition "juvenile-onset Still disease" is now usually grouped under juvenile rheumatoid arthritis. However, there is some evidence that the two conditions are closely related.